A boy, born at term by vaginal delivery, developed coagulopathy and hypovolaemic shock at 15 hours. pH was 6.66,
lactate 21 mmol/l, haemoglobin 8.9 g/dl, platelets 178x10
9
/l, APTT 245 seconds, PT 21 seconds, fibrinogen 0.9 g/l. He
was ventilated and received O-negative blood, plasma, vitamin K, antibiotics and inotropic support. He responded to
treatment and was extubated after eight hours. Coagulation normalised but he required further blood and platelet
transfusions. He developed abdominal distention with hepatomegaly. Ultrasound images suggested a hepatic tumour with
haemorrhage into the tumour and the peritoneal cavity (Figure 1). He required further blood and platelets
transfusions. By day thirteen, the ultrasound appearances were consistent with a resolving hematoma. Repeated
coagulation screens showed prolonged APTT. Factor VIII level of <0.01 IU/ml confirmed a diagnosis of haemophilia A.

A boy, born at term by vaginal delivery, developed coagulopathy and hypovolaemic shock at 15 hours. pH was 6.66,
lactate 21 mmol/l, haemoglobin 8.9 g/dl, platelets 178x10
9
/l, APTT 245 seconds, PT 21 seconds, fibrinogen 0.9 g/l. He
was ventilated and received O-negative blood, plasma, vitamin K, antibiotics and inotropic support. He responded to
treatment and was extubated after eight hours. Coagulation normalised but he required further blood and platelet
transfusions. He developed abdominal distention with hepatomegaly. Ultrasound images suggested a hepatic tumour with
haemorrhage into the tumour and the peritoneal cavity (Figure 1). He required further blood and platelets
transfusions. By day thirteen, the ultrasound appearances were consistent with a resolving hematoma. Repeated
coagulation screens showed prolonged APTT. Factor VIII level of <0.01 IU/ml confirmed a diagnosis of haemophilia A.

en_GB

dc.language.iso

en

en

dc.publisher

Irish Medical Journal

en_GB

dc.subject

NEONATE

en_GB

dc.subject

BLOOD AND IMMUNE DISORDER

en_GB

dc.title

Intra-hepatic haemorrhage and shock during post-natal period, in two brothers with haemophilia

en_GB

dc.type

Article

en

dc.identifier.journal

Irish Medical Journal

en_GB

dc.description.funding

No funding

en

dc.description.province

Leinster

en

dc.description.peer-review

peer-review

en

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